Guillén-López S, Belmont- Martínez L, Fernández-Laínez C, Ibarra-González I, Monroy- Santoyo S, Vela-Amieva M

Language: Spanish
References: 8
Page: 315-318
PDF size: 66.70 Kb.

ABSTRACT

In order to diagnose the nutritional status and to provide a personalized treatment in PKU patients, an evaluation of nutritional status should be conducted. Several components are involved in the nutritional assessment; among the parameters there are anthropometric evaluation: weight, height, head circumference; biochemical: proteins, amino acids, vitamins, inorganic nutrients, essential fatty acids; clinical: deficiencies in hair, skin, oral cavity , eyes, etc.; dietary assessment: quantification of energy intake, macronutrients and micronutrients through several tools such as the 24-hour recall; finally it must be taken into account the interaction of nutrients such as carnitine, fatty acids, vitamin B complex, among others, with drugs, which are often anticonvulsants, in order to plan the diet and prevent a future nutritional deficiency. It is important to perform the nutritional status assessment frequently in patients with PKU because the dietary management is essential to prevent neurological problems.

REFERENCES

1. Acosta P. Evaluation of nutritional status. En: Nutrition Management of Patients with Inherited Metabolic Diseases. Sudbury MA: Jones and Bartlett Pub; 2010. p. 476.

2. Hendricks KM, Duggan C. Eds. Manual pediatric nutrition. 4th Ed. Hamilton, Ontario: BC Decker; 2005. p. 734-7.

3. Mahan K, Escott-Stump S. Nutrición y Dietoterapia de Krause. 12a Ed. España: Elsevier; 2009. p. 223.

4. Francois B, Diels M, de la Brassinne M. Iatrogenic skin lesions in phenylketonuric children due to a low tyrosine intake. J Inherit Metab Dis 1989;12(suppl 2):332-4.

5. Mariotti F, Mahe S, Luengo C, Benamouzig R, Tome D. Postprandial modulation of dietary and whole-body nitrogen utilization by carbohydrates in humans. Am J Clin Nutr 2000; 72: 954-62.

6. Welle S, Matthews DE, Campbell RG, Nair KS. Stimulation of protein turnover by carbohydrate overfeeding in men. Am J Physiol 1989; 257: E413-7.

7. MacDonald A.: Disorders of aminoacide metabolism, organic acidaemias and urea cycle defects. In: Shaw V, Lawson M. Clinical paediatric dietetics. 3rd Ed. UK: Blackwell Pub; 2007. p. 309

8. Singh RH, Quirk ME, Douglas TD, Brauchla MC. BH4 therapy impacts the nutrition status and intake in children with phenylketonuria: 2 year follow-up. J Inherit Metab Dis 2010; 33(6): 689-95.